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Sayyad, et al.: Carcinosarcoma in OHVIRA syndromeMOGS Chronicles | Volume 1 | Issue 1 | September 2024 23DeclarationConflict of interestNone.DisclosureNone.Informed consentInformed consent was taken from the patient.References1. Shavell VI, Montgomery SE, Johnson SC, Diamond MP, Berman JM.Complete septate uterus, obstructed hemivagina, and ipsilateral renal anomaly: Pregnancy course complicated by a rare urogenital anomaly. Arch Gynecol Obstet 2009;280:449-52.2. Oka E, Chiyoda T, Iwata T, Yamagami W, Aoki D. Uterine cervical cancer associated with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome: A case report and review of the literature. Gynecol Oncol Rep 2020;34:100645.3. Banik T, Halder D, Gupta N, Dey P. Malignant mixed M%u00fcllerian tumor of the uterus: Diagnosis of a case by fine-needle aspiration cytology and review of literature. Diagn Cytopathol 2012;40:653-7.4. Bosquet JS, Terstriep SA, Cliby WA, Brown-Jones M, Kaur JS, Podratz KC, et al. The impact of multi-modal therapy on survival for uterine carcinosarcomas. Gynecol Oncol 2010;116:419-23.Editor%u2019s NoteThis rare case of a high-grade vaginal carcinosarcoma in a case of OHVIRA syndrome, highlights the need for increased awareness among clinicians regarding the potential for malignancies in patients with M%u00fcllerian anomalies and the challenges faced in diagnosis and management. Her presentation of persistent midmenstrual bleeding ultimately led to the discovery of a vaginal carcinosarcoma, a malignancy rarely documented in the context of OHVIRA syndrome. It is pertinent to keep vigilance in patients with M%u00fcllerian anomalies, particularly those presenting with atypical symptoms.As the literature on malignancies in OHVIRA syndrome remains limited, this case adds a significant dimension to our understanding. It emphasizes the aggressive nature of the tumor and the importance of early intervention through screening with pap smears, imaging and biopsy protocols.How to cite this article: Sayyad R, Sheriar N, Dharmadhikari N, Sheriar Z. Carcinosarcoma in OHVIRA syndrome: an Enigma.MOGS Chronicles 2024;1(1):21-23.%u00a9 The Author(s). 2024 Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons. org/licenses/by-nc/4.0/), which permits unrestricted use, distribution, and non-commercial reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/ zero/1.0/) applies to the data made available in this article, unless otherwise stated.